2024 ICD-10-CM Diagnosis Code E80.29

Other porphyria

ICD-10-CM Code:
E80.29
ICD-10 Code for:
Other porphyria
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Chronic
Code Navigator:

Code Classification

  • Endocrine, nutritional and metabolic diseases
    (E00–E89)
    • Metabolic disorders
      (E70-E88)
      • Disorders of porphyrin and bilirubin metabolism
        (E80)

E80.29 is a billable diagnosis code used to specify a medical diagnosis of other porphyria. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Adverse effect from PUVA photochemotherapy
  • Chester-type porphyria
  • Complication of hemodialysis
  • Congenital porphyria
  • Coproporphyria
  • Coproporphyrinuria
  • Drug-induced porphyria
  • Drug-induced pseudoporphyria
  • Erythropoietic coproporphyria
  • Ferrochelatase deficiency
  • Heme oxygenase-1 deficiency
  • Hemodialysis-associated pseudoporphyria
  • Hepatic porphyria
  • Hereditary coproporphyria
  • Homozygous hereditary coproporphyria
  • Inherited disorder of porphyrin metabolism
  • Porphobilinogen deaminase deficiency
  • Porphobilinogen synthase deficiency
  • Porphyruria
  • Porphyruria
  • Protoporphyrinuria
  • Pseudoporphyria
  • Pseudoporphyria
  • Pseudoporphyria
  • Pseudoporphyria
  • Pseudoporphyria due to PUVA therapy
  • Skin lesion associated with hemodialysis
  • Uroporphyrinogen decarboxylase deficiency
  • Uroporphyrinuria

Clinical Classification

Clinical Information

  • Acute Intermittent Porphyria|Porphyria, Acute Intermittent

    a genetic metabolic disorder inherited in an autosomal dominant pattern. it is caused by a deficiency of the enzyme porphobilinogen deaminase, which is involved in heme biosynthesis. signs and symptoms include nausea, vomiting, severe abdominal pain and distension, urinary retention, port-wine urine discoloration, hypertension, tachycardia, muscle weakness, loss of sensation, anxiety, depression, and arm, leg and back pain.
  • Bovine Congenital Erythropoietic Porphyria

    a congenital metabolic disorder characterized by a deficiency in the enzyme uroporphyrinogen iii synthase, which occurs in cattle.
  • Bovine Protoporphyria

    a congenital metabolic disorder characterized by a deficiency in the enzyme ferrochelatase, which occurs in cattle.
  • Erythropoietic Porphyria|CEP

    a rare congenital metabolic disorder characterized by an inborn error of porphyrin-heme biosynthesis. it is caused by deficiency of the enzyme uroporphyrinogen iii cosynthetase. it results in cutaneous photosensitivity leading to blistering and scarring of the exposed skin areas.
  • Erythropoietic Protoporphyria|EPP|Protoporphyria, Erythropoietic

    a rare congenital metabolic disorder characterized by an inborn error of porphyrin-heme biosynthesis. it is caused by deficiency of the enzyme ferrochelatase. signs and symptoms include painful cutaneous photosensitivity leading to blistering and scarring of the exposed skin areas, erythrodontia, red discoloration of urine, hemolytic anemia, and splenomegaly.
  • Hepatoerythropoietic Porphyria

    a very rare form of porphyria cutanea tarda. it is characterized by deficiency of the enzyme uroporphyrinogen decarboxylase. signs and symptoms appear early in childhood and include extreme photosensitivity in the sun exposed areas of the skin with blistering and scar formation.
  • Hereditary Coproporphyria

    an autosomal dominant inherited disorder of porphyrin metabolism caused by deficiency of the enzyme coproporphyrinogen oxidase. it results in neurologic damage and can include abdominal pain, constipation and psychiatric manifestations.
  • Porphyria

    a group of genetic or acquired metabolic disorders characterized by defects in the enzymes that are involved in the heme synthesis.
  • Porphyria Cutanea Tarda

    a cutaneous form of the genetic photosensitive disease, porphyria, that is characterized by onset in adult life and the presence of scarring bullae, hyperpigmentation, facial hypertrichosis, and sometimes sclerodermatous thickenings and alopecia. uroporphyrins are found in the urine due to a deficiency of uroporphyrinogen decarboxylase, an enzyme required for the synthesis of heme.
  • Pseudoporphyria

    a drug-induced photodermatosis characterized by skin fragility, erythema, and the appearance of tense bullae, erosions and scarring in the absence of abnormalities in porphyrin metabolism.
  • Variegate Porphyria

    an autosomal dominant disorder of porphyria-heme metabolism. it is manifested with acute attacks including abdominal pain, vomiting, diarrhea, constipation, seizures, anxiety, and confusion. patients may experience skin sensitivity to sunlight.
  • X-Linked Protoporphyria|XLDPP|XLP

    an x-linked dominant porphyria caused by gain of function mutations in the alas2 gene, encoding 5'-aminolevulinate synthase 2 (5-aminolevulinate synthase, erythroid-specific, mitochondrial), which lead to overproduction of protoporphyrin and its accumulation in the blood, liver, and skin. excess protoporphyrin in the blood may lead to iron deficient anemia, while accumulation in the liver may contribute to the formation of gallstones and subsequent obstruction of the bile ducts. exposure to sunlight activates protoporphyrin in the skin, leading to severe pain, burning, and itching.

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.


Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Hereditary coproporphyria

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Convert E80.29 to ICD-9-CM

  • ICD-9-CM Code: 277.1 - Dis porphyrin metabolism
    Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education


Porphyria

Porphyrias are a group of genetic disorders caused by problems with how your body makes a substance called heme. Heme is found throughout the body, especially in your blood and bone marrow, where it carries oxygen.

There are two main types of porphyrias. One affects the skin and the other affects the nervous system. People with the skin type develop blisters, itching, and swelling of their skin when it is exposed to sunlight. The nervous system type is called acute porphyria. Symptoms include pain in the chest, abdomen, limbs, or back; muscle numbness, tingling, paralysis, or cramping; vomiting; constipation; and personality changes or mental disorders. These symptoms come and go.

Certain triggers can cause an attack, including some medicines, smoking, drinking alcohol, infections, stress, and sun exposure. Attacks develop over hours or days. They can last for days or weeks.

Porphyria can be hard to diagnose. It requires blood, urine, and stool tests. Each type of porphyria is treated differently. Treatment may involve avoiding triggers, receiving heme through a vein, taking medicines to relieve symptoms, or having blood drawn to reduce iron in the body. People who have severe attacks may need to be hospitalized.

NIH: National Institute of Diabetes and Digestive and Kidney Diseases


[Learn More in MedlinePlus]

Porphyria

Porphyria is a group of disorders caused by abnormalities in the chemical steps that lead to heme production. Heme is a vital molecule for all of the body's organs, although it is most abundant in the blood, bone marrow, and liver. Heme is a component of several iron-containing proteins called hemoproteins, including hemoglobin (the protein that carries oxygen in the blood).

Researchers have identified several types of porphyria, which are distinguished by their genetic cause and their signs and symptoms. Some types of porphyria, called cutaneous porphyrias, primarily affect the skin. Areas of skin exposed to the sun become fragile and blistered, which can lead to infection, scarring, changes in skin coloring (pigmentation), and increased hair growth. Cutaneous porphyrias include congenital erythropoietic porphyria, erythropoietic protoporphyria, hepatoerythropoietic porphyria, and porphyria cutanea tarda.

Other types of porphyria, called acute porphyrias, primarily affect the nervous system. These disorders are described as "acute" because their signs and symptoms appear quickly and usually last a short time. Episodes of acute porphyria can cause abdominal pain, vomiting, constipation, and diarrhea. During an episode, a person may also experience muscle weakness, seizures, fever, and mental changes such as anxiety and hallucinations. These signs and symptoms can be life-threatening, especially if the muscles that control breathing become paralyzed. Acute porphyrias include acute intermittent porphyria and ALAD deficiency porphyria. Two other forms of porphyria, hereditary coproporphyria and variegate porphyria, can have both acute and cutaneous symptoms.

The porphyrias can also be split into erythropoietic and hepatic types, depending on where damaging compounds called porphyrins and porphyrin precursors first build up in the body. In erythropoietic porphyrias, these compounds originate in the bone marrow. Erythropoietic porphyrias include erythropoietic protoporphyria and congenital erythropoietic porphyria. Health problems associated with erythropoietic porphyrias include a low number of red blood cells (anemia) and enlargement of the spleen (splenomegaly). The other types of porphyrias are considered hepatic porphyrias. In these disorders, porphyrins and porphyrin precursors originate primarily in the liver, leading to abnormal liver function and an increased risk of developing liver cancer.

Environmental factors can strongly influence the occurrence and severity of signs and symptoms of porphyria. Alcohol, smoking, certain drugs, hormones, other illnesses, stress, and dieting or periods without food (fasting) can all trigger the signs and symptoms of some forms of the disorder. Additionally, exposure to sunlight worsens the skin damage in people with cutaneous porphyrias.


[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.